Quality of life among adults with repaired tetralogy of fallot: A literature review.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Survival into adulthood is currently expected following surgical repair, leading to a growing population of adults with repaired TOF. In this literature review, we aim to summarize the current state of knowledge on the quality of life of adults with repaired TOF. A search was conducted on PubMed and results were reviewed for articles published between January of 2010 and June of 2020. Search terms included "Tetralogy of Fallot", "repaired", "adults" and "quality of life". For the subjective health status evaluation, most published studies used Short-Form-36. Most studies agree that physical complex status is poorer for adult patients with repaired TOF than for controls. Mental complex status was also lower. Patients reported similar satisfaction with their lives and levels of social participation. Most patients had a college or university degree. Higher education, male gender and having a partner were positively associated with being employed. Studies found no difference in the proportion of patients that are married or living with a partner, compared to control groups. Patients operated for TOF have a lower reproduction rate compared with the background population. A consistent finding of these studies is abnormal physical parameters compared to psychosocial issues. The diverse needs of adults with repaired TOF require a multidisciplinary care, that takes into consideration all aspects that affect their quality of life. Despite abnormal physical functional status, it is reassuring that most adult patients with TOF lead independent and productive lives.

ROSS SURGERY: OUR EXPERIENCE.

OBJECTIVES: At Santa Marta Hospital, Ross Surgery was performed for the first time in 1999. Twenty years later, we feel it is desirable to evaluate the mid and long-term results of our experience, as well as estimate the future of this procedure. METHODS: Between March 1999 and June 2016, 23 Ross procedures were performed at our institution. We did a retrospective analysis of the patients´ data, results of the surgery, complications, freedom from reoperation and mortality. RESULTS: The majority (36,4%) of the patients had aortic stenosis, 22,7% had aortic regurgitation and 27,3% had aortic stenosis and regurgitation. Sub-valvular stenosis was present in 13,6% of patients. The mean follow-up is 15 years. The overall mortality was 9%, without early mortality. In our series, 83% of the patients are free from reoperation. Eighty percent (n=16) of the survivors are in NYHA class I, with the remaining 20% (n=4) in class II. CONCLUSION: Ross surgery has strict indications and in this group of patients the advantages are undeniable and the outcomes, according to our results (with 83% of patients free from reoperation, at a mean follow-up of 15 years), are positive and encouraging.

SURGICAL ABLATION OF ATRIAL FIBRILLATION AND LEFT ATRIAL APPENDAGE OCCLUSION BY A TOTALLY VIDEOTHORACOSCOPIC APPROACH - NEW PARADIGM?

INTRODUCTION: Atrial fibrillation (AF) contributes to increased morbidity and mortality. Pharmacological and percutaneous catheter therapies are unsatisfactory, with potential serious adverse effects. Cox-Maze III/IV surgery, with higher rates of success, has not been widely adopted because of the associated complexity of the procedure. METHODS: We performed a retrospective analysis of the first patients submitted to surgical ablation of AF with occlusion of the left atrial appendage with a totally videothoracoscopic (VATS) approach in our institution. We describe the surgical technique and our results, including duration of surgery, hospital stay, complications and maintenance of sinus rhythm after surgery, at 6, 12 and 18 months of follow-up. RESULTS: We studied 15 patients (ages ranging from 39 to 75 years old; 54,5% female gender). Mean time since the diagnosis of AF was 5,75 years. All had been submitted to prior catheter ablation (mean of 2 attempts). Mean diameter and volume of the left atrium was 42 mm (M-mode) and 70 ml (43 ml/m2), respectively. Mean duration of surgery was 2 hours and 22 minutes. In one patient we had to convert the surgery to median sternotomy. Mean hospital stay was 4,8 days. Mean time of follow-up was 12 months. During follow-up, 91%, 90% and 80% of the patients were in sinus rhythm at 6, 12 and 18 months, respectively. CONCLUSION: This surgical approach represents a real benefit for those patients with multiple attempts of catheter ablation without success. However, a larger sample of patients with a longer period of follow-up is necessary for further conclusions.

Arterial Switch Operation Variables Predicting Reoperation.

OBJECTIVES: Jatene surgery or arterial switch is performed at our institution since the late nineties. We reviewed our results to identify the main causes of reoperation and, more importantly, to determine what variables predict the need for reoperation. METHODS: In this retrospective analysis were included all the 91 patients with d-TGA who underwent an arterial switch operation at our institution between 1995 and 2016. RESULTS: Mean follow-up was 10 years (range 5-25 years). Seventy-one percent of patients had simple TGA and 29% had complex TGA. The need of reoperation was 21% (n=19 patients). Right ventricle outflow tract obstruction was the main indication for reoperation (58%). The overall mortality was 9.9%. The gender (P= 0.8), diagnosis (simple or complex TGA) (P= 0,5) or the existence of palliative surgeries (P=0.9) were unable to predict the need for reoperation. The presence of anomalous coronary pattern was the only variable reaching statistical significance (P < 0.05), both in univariate and multivariate analysis. CONCLUSIONS: In our series, the main indication for reoperation after arterial switch operation was right ventricle outflow tract obstruction and the only predictive variable was the presence of anomalous coronary pattern.

Right Ventricular Outflow Tract Pseudo Aneurysm After Reconstruction With Small Intestinal Submucosal Cormatrix Patch A Word Of Caution.

Tetralogy of Fallot is very prevalent with correction techniques well standardized. Whenever infundibular incisions are needed, patch reconstruction seems mandatory. Recently, the small intestinal submucosal (CorMatrix, MAC's Medical Group,) patch was introduced, with optimal results in pre-clinical studies. However, clinical results do not match its pre-clinical promises, particularly when used in right ventricular outflow tract and pulmonary artery reconstructions. We describe a case of Tetralogy of Fallot for which small intestinal submucosal (CorMatrix) patch was used as a trans-annular patch, with development of a massive pseudo-aneurysm.

A Very Rare Case of Coronary Artery Bypass Grafting in a Progeria Child.

Hutchinson-Gilford progeria syndrome is a rare genetic disorder, characterized by progressive premature aging and early death in the first or second decade of life, usually secondary to cardiovascular events (myocardial infarction and stroke). We report a case of a 14-year-old boy with progeria syndrome and cardiac arrest due to myocardial infarction, who was submitted to an immediate coronary angiography which revealed left main stem and three-vessel coronary artery disease. A prompt double bypass coronary artery grafting surgery was performed, and, despite successful coronary reperfusion, the patient remained in coma and brain death was declared on fourth day after surgery.

Isolated Tricuspid Valve Surgery: Repair Versus Replacement.

INTRODUCTION: Isolated tricuspid valve surgery (ITVS) is an uncommon procedure with few studies published. We report our series of ITVS and compare surgical outcomes and mortality in patients undergoing valve repair (TVR) versus replacement (TVRep). METHODS: Retrospective study including all patients who underwent ITVS (n = 34) between July 2008 and June 2017, divided in two groups according to type of procedure: TVR 20 patients and TVRep 14 patients. We reviewed preoperative characteristics and analysed operative data, outcomes and mortality in both groups. RESULTS: Thirty-four patients underwent ITVS, mean age 58,1±15,9 years, 50% female and mean BMI 26,1kg/ m2. TVR was performed in 58,8% and TVRep in 41,2% of patients. Patients had similar demographic and baseline characteristics, except for previous cardiac surgery (TVRep 78,6% vs.TVR 35,%, p<0,05). Mean logistic EuroSCORE was 10,1% for TVRep and 6,6% for TVR (p<0,05). Etiologies were functional insufficiency (68%), endocarditis (18%), degenerative (9%), rheumatic (3%) and congenital (3%). TVR was the preferred surgical approach. Ring annuloplasty was performed for all TVR and bioprosthesis was used for all TVRep. Postoperative complications were: need for transfusional support (76,5%), inotropic support longer than 48 hours (38,2%), prolonged invasive ventilation over 24 hours (35,3%), new onset of atrial fibrillation (11,8%), duplication or postoperative creatinine over 2 mg/dl (8,8%), dialysis (8,8%), stroke (5,9%), intra-aortic balloon pump (5,9%), permanent pacemaker implantation (2,9%) and sepsis (2,9%). TVRep was associated with superior length of surgery (TVRep 291 vs. TVR 186 minutes), longer ICU stay (TVRep 17,1 vs.TVR 2,8 days), longer hospital stay (TVRep 37,1 vs.TVR 11,7 days), prolonged invasive ventilation (TVRep 71,4% vs.TVR 10%) and longer inotropic support (TVRep 78,6% vs.TVR 10%) (p<0,05). Overall 30-day and 1-year mortality were 8,8% and 17,6%, respectively. Type of procedure was not associated to 30-day mortality (TVRep 14,3% vs. TVR 5%, p<0,05), but TVRep was associated with higher 1-year mortality (TVRep 35,7% vs. TVR 5%, p<0,05). CONCLUSIONS: TVR showed better outcomes, with less postoperative complications and mortality than TVRep. This difference cannot be linked to type of pathology, although patients in the latter group may be associated to greater complexity, with higher risk and were often reoperations.

Arterial Switch: How to Predict Reoperation.

INTRODUCTION: Jatene surgery or arterial switch is performed at our institution since 1989. It is mandatory to submit our results to an evaluation that allow us to identify the main causes of reoperation and, more importantly, to determine what variables predict the need of reoperation. METHODS: In this retrospective analysis were included all the 91 patients with d-TGA who underwent an arterial switch operation at Santa Marta Hospital between 1999 and 2016. RESULTS: Mean follow-up was 6 years (range 1-21 years). 71% of the patients had simple TGA and 29% had complex TGA. The need of reoperation was 21%(n=19). Pulmonary artery stenosis was the main (47%) indication for reoperation. The overall cumulative mortality was 9.9%. The gender (P= 0.8), diagnosis (simple or complex TGA) (P= 0,5) or the existence of previous surgeries(P=0.9) were unable to predict the need of reoperation. The presence of coronary patterns anomalies was the only variable reaching statistical significance (P< 0.05), both in univariate and multivariate analysis. CONCLUSION: In our series, the main indication for reoperation after arterial switch operation was pulmonary artery stenosis and the only predictive variable was the presence of coronary anomalies.

Ross Surgery: Our Experience.

INTRODUCTION: At Santa Marta Hospital, Ross Surgery was performed for the first time in 1999. Seventeen years later, it is mandatory to evaluate the mid and long-term results of our experience, as well as prospect the future of this procedure. METHODS: Between March 1999 and June 2016, 23 Ross procedures were performed at our institution. We did a retrospective analysis of the patient's data, results of the surgery, complications, freedom from reoperation and mortality. RESULTS: The majority (36,4%) of the patients had aortic stenosis, 22,7% had aortic regurgitation and 27,3% had aortic stenosis and regurgitation. Sub-valvular stenosis was present in 13,6% of patients. The mean follow-up is 12 years. Eighty percent (n=16) of the survivors are in class I of NYHA, with the remaining 20% (n=4) in class II. In our series, 81% of the patients are free from reoperation. The overall cumulative mortality was 9%. There was no operative mortality. CONCLUSION: Ross surgery has specific indications and in this group of patients the advantages are undeniable and the results, according to our series are positive and encouraging.

The influence of alignment on risk of knee osteoarthritis progression according to baseline stage of disease.

OBJECTIVE: Varus and valgus malalignment increase the risk of medial and lateral osteoarthritis (OA) progression, respectively. The impact of a mechanical factor such as alignment depends not only on the factor itself, but also on the state of the joint. The less-damaged joint of mild OA may be less vulnerable to malalignment effects than the more-damaged joint of moderate OA. Our goal was to explore the impact of alignment on subsequent progression of knee OA according to the baseline stage of disease. METHODS: Two hundred thirty patients with knee OA (defined by the presence of osteophytes and symptoms) recruited from the community underwent assessment of both lower limbs at baseline and at an 18-month followup. Alignment was measured on a full-limb radiograph as the angle made by the intersection of the femoral and tibial mechanical axes. Compartment-specific progression was defined as an increase between baseline and 18 months in the grade of severity of joint space narrowing on radiographs of semiflexed knees taken after fluoroscopic confirmation of position. Knees were grouped according to their baseline stage of OA as Kellgren/Lawrence (K/L) grades 0-1, 2, or 3. Progression odds ratios were estimated from logistic regression using generalized estimating equations. RESULTS: There were 377 K/L grade 0-3 knees in 230 subjects (173 women and 57 men, mean age 64.0 years, mean body mass index 30.4 kg/m(2)) in this longitudinal study. In knees with mild OA (K/L grade 2), the odds of 18-month progression in the medial compartment were significantly increased 4-fold by varus alignment at baseline. In K/L grade 2 knees, the odds of lateral progression were increased 2-fold by valgus alignment (approaching significance). In knees with moderate OA (K/L grade 3), the risk of progression was comparably increased by varus or valgus alignment (10-fold). CONCLUSION: While some effect of malalignment was suggested at almost all stages of knee OA examined, the impact of varus or valgus malalignment on the odds of OA progression over the ensuing 18 months was greater in knees with moderate (K/L grade 3) OA at baseline, possibly due to greater joint vulnerability with some contribution from slightly more severe malalignment.